Objectives To assess the performance of three risk scores from Japan that were developed to predict, in children with Kawasaki disease, resistance to intravenous immunoglobulin (IVIG) treatment. Study design We used data from a randomized trial of pulsed steroids for primary treatment of Kawasaki disease to assess operating characteristics of the three risk scores, and we examined whether steroid therapy lowers the risk of coronary artery abnormalities in patients prospectively classified as IVIG resistant. Results For comparability with published cohorts, we analyzed the data of 99 patients not treated with steroids (16% IVIG-retreated), and identified male sex, lower albumin and higher AST as independent risk factors for IVIG resistance. The Kobayashi score was similar in IVIG-resistant and responsive patients, yielding sensitivity=33% and specificity=87%. There was no interaction of high vs. low risk status by treatment received (steroid vs. placebo) using any of the three risk score algorithms. Conclusion Risk scoring systems from Japan have good specificity but low sensitivity for predicting IVIG resistance in a North American cohort. Primary steroid therapy did not improve coronary outcomes among patients prospectively classified as high risk for IVIG resistance.

SummaryBackground Kawasaki disease, the most common cause of acquired heart disease in developed countries, is a self-limited vasculitis that is treated with high doses of intravenous immunoglobulin. Resistance to intravenous immunoglobulin in Kawasaki disease increases the risk of coronary artery aneurysms. We assessed whether the addition of infl iximab to standard therapy (intravenous immunoglobulin and aspirin) in acute Kawasaki disease reduces the rate of treatment resistance.

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Background-Cardiovascular pathology, including aortic root dilation, dissection, and rupture, is the leading cause of mortality in patients with Marfan syndrome (MFS). The maximal aortic root diameter at the sinuses of Valsalva is considered the best predictor of adverse cardiovascular outcome. Although advances in therapy have improved life expectancy, affected individuals continue to suffer cardiovascular morbidity and mortality. Recent studies in a FBN1-targeted mouse model of MFS with aortic pathology similar to that seen in humans showed that treatment with losartan normalized aortic root growth and aortic wall architecture.